Friedreich's ataxia electrocardiogram

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: MMJ

Overview

An ECG may be helpful in the diagnosis of Friedreich's ataxia. Findings on an ECG suggestive of Friedreich's ataxia include: T-wave repolarization abnormalities especially in the inferior and lateral leads, Bundle branch blocks, ventricular hypertrophy, right axis deviation, short PR interval, interatrial block, left atrial enlargement and right atrial enlargement.

Electrocardiogram

An ECG may be helpful in the diagnosis of Friedreich's ataxia. Findings on an ECG suggestive of Friedreich's ataxia include:[1][2][3][4][5][6]

  • T-wave repolarization abnormalities, especially in the inferior and lateral leads, is the most common abnormalities seen on the electrocardiogram (ECG) of patients with Friedreich's ataxia(approximately 85%).
  • Bundle branch blocks is found in 15% of patients with Friedreich's ataxia

References

  1. Hanson E, Sheldon M, Pacheco B, Alkubeysi M, Raizada V (January 2019). "Heart disease in Friedreich's ataxia". World J Cardiol. 11 (1): 1–12. doi:10.4330/wjc.v11.i1.1. PMC 6354072. PMID 30705738.
  2. Payne RM, Wagner GR (September 2012). "Cardiomyopathy in Friedreich ataxia: clinical findings and research". J. Child Neurol. 27 (9): 1179–86. doi:10.1177/0883073812448535. PMC 3674565. PMID 22764179.
  3. Pousset F, Legrand L, Monin ML, Ewenczyk C, Charles P, Komajda M, Brice A, Pandolfo M, Isnard R, Tezenas du Montcel S, Durr A (November 2015). "A 22-Year Follow-up Study of Long-term Cardiac Outcome and Predictors of Survival in Friedreich Ataxia". JAMA Neurol. 72 (11): 1334–41. doi:10.1001/jamaneurol.2015.1855. PMID 26414159.
  4. Payne RM, Peverill RE (December 2012). "Cardiomyopathy of Friedreich's ataxia (FRDA)". Ir J Med Sci. 181 (4): 569–70. doi:10.1007/s11845-012-0808-7. PMID 22373590.
  5. Weidemann F, Störk S, Liu D, Hu K, Herrmann S, Ertl G, Niemann M (August 2013). "Cardiomyopathy of Friedreich ataxia". J. Neurochem. 126 Suppl 1: 88–93. doi:10.1111/jnc.12217. PMID 23859344.
  6. Schadt KA, Friedman LS, Regner SR, Mark GE, Lynch DR, Lin KY (September 2012). "Cross-sectional analysis of electrocardiograms in a large heterogeneous cohort of Friedreich ataxia subjects". J. Child Neurol. 27 (9): 1187–92. doi:10.1177/0883073812448461. PMC 3674639. PMID 22752487.

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